Pathology of a new Toxic Syndrome caused by ingestion of adulterated oil in Spain

Summary The Toxic Syndrome (TS) caused by ingestion of adulterated rapeseed oil in Spain is a new disease of multisystemic character whose aetiology and pathogenesis remains unknown. The most prominent pathological feature is a peculiar non-necrotizing vasculitis, that affects mainly the intima and involves vessels of every type and size in practically every organ. The TS begins with an acute clinical picture with pleuropneumopathy, fever, headaches, exanthems and eosinophilia. In these early clinical phases the main pathological findings were observed in the lungs and consisted of intense pulmonary interstitial oedema with scanty inflammatory mononuclear infiltrates. Ultrastructural study revealed hydropic degeneration of pneumocytes types I and II with desquamation of type I. The patients in this phase died of respiratory failure, later deaths were due to thromboembolic complications. Later still the patients developped a neuromuscular syndrome, sclerodermiform skin lesions and severe weight loss and died predominantly of infectious complications and respiratory failure. The anatomopathological picture in the peripheral nerves was that of inflammatory neuropathy with a lymphocytic perineuritis that led to perineural fibrosis with secondary axonal degeneration. The muscle presented an interstitial inflammatory myopathy at first followed by a neurogenic muscular atrophy. The skin lesions in the late phases consisted in dermal or dermal and subdermal fibrosclerosis, with vasculitis of the small arteries in the lower dermis. The salivary glands and pancreas showed vasculitis and interstitial inflammation which progressed to interstitial fibrosis and parenchymal atrophy.     

Apoptosis of perineurial cells in sensory perineuritis

A 71-year-old man presented a 6-month history of progressive paresthesia of all four limbs. Sural nerve biopsy specimens showed dense mononuclear infiltrates in the perineurium and subperineurium, indicating sensory perineuritis. One section revealed disruption of the perineurial barrier. Perforin and granzyme B were present in the infiltrates, and apoptosis of perineurial cells was indicated by a terminal deoxynucleotidyl-transferase-mediated dUTP-digoxigenin nick end-labeling (TUNEL) method. These findings suggest T cell-mediated apoptosis of the perineurium and nerve injury caused by perineurial damage. Received: 5 May 1999 / Revised, accepted: 12 July 19993     

Non specific orbital inflammatory diseases

The understanding of pseudotumors has significantly changed with the advent of modern imaging techniques by which the tissues affected in the orbit can be identified, and nonspecific orbital inflammatory disease is classified according to the tissue involved. Twenty cases of nonspecific orbital inflammatory diseases were treated between July 1987 and September 1991. Eight patients had myositis, 6 had diffuse disease, 2 each had dacryoadenitis, periscleritis and perineuritis. Involvement of all four recti in one patient and isolated superior rectus and superior oblique each in 2 others are the unusual forms of myositis. Perineuritis presented as diffuse thickening of the optic nerve on CT scan with disc edema and normal vision in both patients with external ophthalmoplegia in one of them. All patients responded to oral steroids while the patient with myositis of all four recti required radiotherapy in addition. This series documents the spectrum of clinical presentation, diagnosis and management of nonspecific orbital inflammatory disease.